CARDIAC TROPONINS AND N-TERMINAL PRO-BRAIN NATRIURETIC PEPTIDE PREDICT SURVIVAL IN PATIENTS WITH PRIMARY SYSTEMIC AMYLOIDOS1S UNDERGOING PERIPHERAL BLOOD STEM CELL TRANSPLANT

摘要

Primary systemic amyloidosis (AL) is a disease without cure. Pilot data suggest survival is better in patients undergoing peripheral blood stem cell transplant (PBSCT), but the selection process makes the apparent benefit suspect. We have reported that circulating cardiac biomarkers are the best predictors of survival outside of the transplant setting. We now test whether cardiac troponins (cTnT and cTnl) and N-terminal pro-brain natriuretic peptide (NT-proBNP) are prognostic in transplant patients. Ninety-eight patients with AL undergoing PBSCT had serum cardiac biomarkers measured (cTnT, 98; cTnl, 65; and NT-proBNP, 63 patients). The troponin and NT-proBNP values and scores of these patients were compared to those of our previously reported cohort of 242 patients not undergoing transplant. Elevated levels of cTnT, cTnl, NT-proBNP were present in 14%, 43%, and 48%. Median survival has not been reached for patients with values below the thresholds at 20 months and if above is 26.1, 66.1 and 66.1 months, respectively. Our previously reported risk and staging systems incorporating these markers were also prognostic. Despite the observation that the PBSCT group was a significantly lower risk group than our previously reported non-transplant group, PBSCT was associated with superior survival after correction for risk or stage.